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Energy Requirements in Mitochondrial Disease

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ClinicalTrials.gov Identifier: NCT01776918
Recruitment Status : Completed
First Posted : January 28, 2013
Last Update Posted : July 18, 2017
Sponsor:
Collaborator:
Rare Disease Foundation, Vancouver, Canada
Information provided by (Responsible Party):
Rajavel Elango, PhD, University of British Columbia

January 17, 2013
January 28, 2013
July 18, 2017
February 2012
June 2014   (Final data collection date for primary outcome measure)
Resting Energy Expenditure [ Time Frame: 1 hour ]
Resting Energy Expenditure will be measured by carbon dioxide production and oxygen consumption.
Same as current
Complete list of historical versions of study NCT01776918 on ClinicalTrials.gov Archive Site
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Energy Requirements in Mitochondrial Disease
Energy Requirements in Metabolic and Mitochondrial Disease

Metabolic diseases and mitochondrial disorders are caused by genetic mutation which lead to disruptions in energy producing pathways in our body. Enough energy or calories must be given in the diet to ensure normal growth and development. Currently, energy needs for patients with metabolic and mitochondrial diseases are not measured, but is estimated using a mathematical equation based on healthy children. This may lead to under feeding or overfeeding of calories, and has negative nutritional implications.

The clinical standard for measuring energy needs is the use of indirect calorimeter.The indirect calorimeter takes individualized measurements for each patient and therefore will enable dietitians and clinicians to provide sufficient calories in the diet to better manage the disease and promote normal growth and development.

We believe daily energy requirements will vary within metabolic diseases (Phenylketonuria) and mitochondrial disorders (mitochondrial fatty acid oxidation defect, POLG1 mutation etc.).

The objective of this preliminary study is to measure resting energy expenditure in children living with metabolic and mitochondrial conditions and data obtained will be used to generate future hypothesis and will form a basis for future studies.

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Observational
Observational Model: Case-Only
Time Perspective: Cross-Sectional
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Non-Probability Sample
Children (1-18y) diagnosed with either phenylketonuria or a mitochondrial disorder
  • Mitochondrial Disease
  • Chronic Metabolic Disorder
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  • Metabolic Disease- Phenylketonuria
  • Mitochondrial disorder
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*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Completed
23
20
September 2014
June 2014   (Final data collection date for primary outcome measure)

Inclusion Criteria:

  • Children(1-18y) who are diagnosed with either Phenylketonuria (PKU) or a mitochondrial disorder

Exclusion Criteria:

  • Children, less than 1y old, who are diagnosed with PKU or a mitochondrial disorder, as it is difficult to perform indirect calorimeter on them.
  • Children(1-18y) who are not diagnosed with PKU or a mitochondrial disorder
  • Children(1-18y) who are diagnosed with either PKU or a mitochondrial disorder, but are currently experiencing illness such as fever, cold, vomiting or diarrhea
Sexes Eligible for Study: All
1 Year to 18 Years   (Child, Adult)
No
Contact information is only displayed when the study is recruiting subjects
Canada
 
 
NCT01776918
H11-02094
No
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Rajavel Elango, PhD, University of British Columbia
University of British Columbia
Rare Disease Foundation, Vancouver, Canada
Principal Investigator: Rajavel Elango, PhD Child & Family Research Institute/University of British Columbia
Study Chair: Sylvia Stockler-Ipsiroglu University of British Columbia/Faculty of Pediatrics
Study Chair: Ramona Meni Salvarinova Zivkovic University of Bristish Columbia/Faculty of Pediatrics
Study Chair: Howard Parsons University of British Columbia/Faculty of Pediatrics
University of British Columbia
July 2017